Improving the detection of pulmonary hypertension in systemic sclerosis using pulmonary function tests

Abstract Background Systemic sclerosis (SSc) is a multi-system connective tissue disease characterized by microvascular damage, inflammation and fibrosis. The aim of our study is to investigate a possible correlation between nailfold videocapillaroscopy patterns and internal organ involvement in patients with early SSc. Methods We enrolled 40 patients with early SSc according to LeRoy criteria and performed echocardiogram, pulmonary function tests (PFTs) and nailfold videocapillaroscopy. Results 20 patients had early pattern and 20 active pattern. A comparative analysis between SSc patients and healthy controls showed an alteration of mitral annular plane systolic excursion (MAPSE) (1.5±0.1 vs 1.7±0.1; p < 0.001), respectively; an increased occurrence of diastolic dysfunction (11/40 vs 0/21; p = 0.01) respectively; a higher values of systolic pulmonary arterial pression (PAPs) (32±6 vs 22±6; p < 0.001) and a higher frequency of patients with pulmonary hypertension (12/40 vs 0/21; p = 0.005) in SSc group. The patients with an active capillaroscopic pattern showed a trend of reduction in MAPSE, diastolic dysfunction, contractility of the right ventricle and pulmonary function tests parameters. We observed a correlation between myocardial and pulmonary vascular damage and between myocardial and pulmonary fibrosis, with lower DLCO value in patients with diastolic dysfunction (64±17 vs 85±9; p < 0.0001). Conclusion A subclinical myocardial damage in these patients may be observed also in the absence of symptoms. In fact, in our study it can be noted that asymptomatic patients present a reduction in the linear myocardial contractility. Furthermore, we observed the presence of grade I diastolic dysfunction in 27.5% of cases, a sign of an initial stiffening of the wall, likely due to a fibrotic damage. In our study linear contractility emerges to be not correlated with the presence of diastolic dysfunction. Such data is very intriguing if we consider that in many studies the resolution of linear contractility with conserved ejection fraction correlated with subendocardial damage induced by microcirculation alterations. The hypothesis is the presence of a double framework at the myocardial level: a vascular damage and the presence of increased inflammation and fibrosis. The presence fibrosis is correlated between the various organs, with the values of DLCO strongly correlating with the presence of diastolic dysfunction; instead DLCO values do not correlate with pulmonary pressure values. The analysis of the capillaroscopic parameters we observed how in the early pattern the presence of vascular damage with MAPSE and PAPs alterations and presence of pulmonary hypertension emerge, while passing from early to active the presence of fibrotic damage becomes evident, the diastolic dysfunction passing from 15% to 40% and DLCO passing from 83% to 75%. The presence of subclinical cardiac and pulmonary damage in patients with systemic sclerosis and that this damage is closely related to the capillaroscopic pattern. Disclosures F. Masini: None. L. Monaco: None. K. Gjeloshi: None. E. Pinotti: None. R. Ferrara: None. T. Salvatore: None. G. Cuomo: None.

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Pulmonary hypertension after ventriculoatrial shunt implantation

Journal of Neurosurgery ◽

10.3171/2010.6.jns091541 ◽

2010 ◽

Vol 113 (6) ◽

pp. 1279-1283 ◽

Cited By ~ 22

Author(s):

Stefan Kluge ◽

Hans Jörg Baumann ◽

Jan Regelsberger ◽

Uwe Kehler ◽

Jan Gliemroth ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Pulmonary Function ◽

Pulmonary Function Tests ◽

Significant Proportion ◽

Systolic Pressure ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Doppler Velocity ◽

Systolic Pulmonary Artery Pressure ◽

Function Tests

Object Ventriculoatrial (VA) shunts inserted for the treatment of hydrocephalus are known to be a risk factor for pulmonary hypertension. The aim of this study was to evaluate the incidence of pulmonary hypertension among adult patients with VA shunts. Methods All patients who had received a VA shunt at one of two institutions between 1985 and 2000 were invited for a cardiopulmonary evaluation. The investigation included a thorough history taking, clinical examination, echocardiography, and pulmonary function testing including diffusing capacity of the lung for carbon monoxide (DLCO). Pulmonary hypertension was defined as systolic pulmonary artery pressure > 35 mm Hg at rest. Results The study group consisted of 86 patients, of whom 38 (44%) could be examined. The patients' mean age was 47.1 ± 18.4 years; the median interval between shunt insertion and cardiopulmonary evaluation was 15 years (range 5–20 years). Of the 38 patients, 20 (53%) had Doppler velocity profiles of tricuspid regurgitation that were adequate for the estimation of pulmonary artery systolic pressure. Doppler-defined pulmonary hypertension was observed in 3 patients (8%), 2 of whom underwent right heart catheterization. Chronic thromboembolic pulmonary hypertension was confirmed in both patients, and medical therapy, including anticoagulation, was started. The VA shunt was removed in both cases and replaced with a different type of device. Pulmonary function tests revealed a restrictive pattern in 15% and typical obstructive findings in 9% of patients. In 30% of patients the DLCO was less than 80% of predicted, and blood gas analysis showed hypoxemia in 6% of patients. No significant differences in pulmonary function tests were noted between the patients with and without echocardiographic evidence of pulmonary hypertension. However, patients with pulmonary hypertension had significantly lower DLCO values. Conclusions The authors detected pulmonary hypertension by using Doppler echocardiography in a significant proportion of patients with VA shunts. It is therefore recommended that practitioners perform regular echocardiography and pulmonary function tests, including single-breath DLCO in these patients to screen for pulmonary hypertension to prevent hazardous late cardiopulmonary complications.

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Longitudinal change during follow-up of systemic sclerosis: correlation between high-resolution computed tomography and pulmonary function tests

Clinical Rheumatology ◽

10.1007/s10067-020-05375-y ◽

2020 ◽

Vol 40 (1) ◽

pp. 213-219

Author(s):

Aldo Carnevale ◽

Mario Silva ◽

Elisa Maietti ◽

Gianluca Milanese ◽

Marta Saracco ◽

...

Keyword(s):

Computed Tomography ◽

Systemic Sclerosis ◽

High Resolution ◽

Pulmonary Function ◽

Pulmonary Function Tests ◽

Longitudinal Change ◽

High Resolution Computed Tomography ◽

Function Tests

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HRCT and pulmonary function tests in monitoring of lung involvement in juvenile systemic sclerosis

Pediatric Pulmonology ◽

10.1002/ppul.21141 ◽

2009 ◽

Vol 44 (12) ◽

pp. 1226-1234 ◽

Cited By ~ 17

Author(s):

Serena Panigada ◽

Angelo Ravelli ◽

Michela Silvestri ◽

Claudio Granata ◽

Silvia Magni-Manzoni ◽

...

Keyword(s):

Systemic Sclerosis ◽

Pulmonary Function ◽

Pulmonary Function Tests ◽

Lung Involvement ◽

Function Tests ◽

Juvenile Systemic Sclerosis

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Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease

European Respiratory Review ◽

10.1183/16000617.0102-2017 ◽

2018 ◽

Vol 27 (148) ◽

pp. 170102 ◽

Cited By ~ 15

Author(s):

Melissa Caron ◽

Sabrina Hoa ◽

Marie Hudson ◽

Kevin Schwartzman ◽

Russell Steele

Keyword(s):

Systematic Review ◽

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Validation Studies ◽

Pulmonary Function Tests ◽

Outcome Studies ◽

Surrogate Markers ◽

Function Tests

Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression (i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD (i.e. validation studies).This systematic review included 169 outcome studies and 50 validation studies. Diffusing capacity of the lung for carbon monoxide (DLCO) was cumulatively the most commonly used outcome until 2010 when it was surpassed by forced vital capacity (FVC). FVC (% predicted) was the primary endpoint in 70.4% of studies, compared to 11.3% for % predicted DLCO. Only five studies specifically aimed to validate the PFTs: two concluded that DLCO was the best measure of SSc-ILD extent, while the others did not favour any PFT. These studies also showed respectable validity measures for total lung capacity (TLC).Despite the current preference for FVC, available evidence suggests that DLCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression.

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